One Family’s Journey:
Early Intervention Paves the Way
By Joelle Roskens, South Dakota H&V
It’s hard to believe that it has been seven years since our oldest daughter, Berkley, was born. It seems like yesterday we were bringing our bundle of joy home for the first time. As she was our first, we were very excited to begin our journey as first-time parents. I remember clearly as we were walking out of our hospital room, the nurse stopped us in the doorway to give us some additional paperwork. That was the first time we were notified that Berkley had “failed” her newborn hearing screening. We were advised to follow up with audiology, but not to worry, as she “more than likely had fluid in her ears”.
Two weeks later, Berkley was evaluated by audiology and was diagnosed with bilateral severe to profound sensorineural hearing loss. Three weeks later, she was fit with her first pair of hearing aids. The following weeks were a blur as there was much to do. We were busy scheduling doctor appointments, contacting our local school for the deaf and birth to three program, researching hearing loss and trying to learn sign language to communicate with our daughter–all on top of being new parents. It was quite overwhelming, but we knew we had to do what was best for our child. Every website we viewed stated that early detection and intervention was key. And, boy, were they ever right!
Finding our Way
It took us quite some time to settle into our “new normal”. As an infant, Berkley had quite the busy schedule. We had weekly in-home visits with her deaf educator and outreach consultant, family signing classes and multiple trips to the Mayo Clinic in Rochester, MN for her audiology appointments. I learned along the way how much deaf and hard of hearing children could miss out on incidental learning experiences. I felt like I never stopped talking or signing and explained every little detail as to what was on going around us.
After a lot of research and thought about what would be best for our daughter, we decided on cochlear implants. While she was approved early, she was found to have excessive fluid at 7 months old and PE tubes were placed. She underwent bilateral implant surgery at ten months of age at Mayo Clinic. Her Advanced Bionics processors were activated two weeks later with success. After activation, we added more supports, including twice-weekly speech therapy sessions and twice-weekly visits with her deaf educator.
Genetics Results
We learned a few months after surgery that Berkley’s hearing loss was due to a genetic mutation. Connexin 26 is a protein found on the GJB2 gene and is the most common cause of congenital sensorineural hearing loss. Connexin 26 mutations are also responsible for at least 20% of all genetic hearing loss and 10% of all childhood hearing loss. Connexin 26 mutations are genetically transmitted from parent to child in a recessive manner, which means that an affected individual must inherit one copy of the gene from each parent. We were expecting a second child when we learned about her genetic testing results. We had already decided that we could handle the challenge of another child with hearing loss if faced with a similar situation.
By kindergarten, Berkley was in a mainstreamed school and excelling across the board. In first grade, after a team assessment, she lost all of her services, including weekly speech therapy and deaf education, and her IEP was removed. (She does have accommodations, such as her FM system, preferential seating, etc. but we don’t have a formal 504 Plan yet.) What if she gets behind? What if she isn’t getting what she is supposed to? Why on earth doesn’t she qualify for deaf education as a deaf child? What about ASL interpreting? There are still so many unanswered questions with regard to the quality education every child needs, let alone a deaf child. As parents, we were not thrilled to lose the very services that contributed to her success thus far. We will be carefully watching her progress, her inclusion, and continuing with private therapy to make sure she stays on track. We keep in close contact with her teacher and are in the process of trying to get her back on an IEP as we learned that doing well academically is not a reason on its own to remove a child from special education.
With all that said, Berkley is currently in 2nd grade and is a remarkable student. Berkley loves school and continues to amaze us every day. She loves to read, write and is a math whiz. That little girl will move mountains one day! One thing that makes us the happiest as parents is that she is so proud to be deaf. She loves to teach her sister, Charley, how to sign everything!
Although there were many ups and downs, I am very thankful for our personal family experience. It has led us to such wonderful people and great opportunities. As a registered nurse in a pediatric specialty clinic, along with being one of the four directors of the South Dakota-Hands & Voices chapter, I have the opportunity to make a difference and provide resources and support to parents with children who are deaf and hard of hearing. ~
H&V Communicator – Winter 2021